Background/Purpose: There have been three classification proposals for Sjögren´s Syndrome (SS) over the past 15 years (1-3). The 2016 criteria require a diagnostic work-up which includes at least evaluation of focus score (FS) in minor salivary gland (MSG) biopsy, anti-SSA antibodies, Schirmer’s test, unstimulated whole salivary flow (UWS), and ocular staining score (OSS), the last two tests being unavailable at our centre. Patients have to score ≥4 points to fulfill the ACR-EULAR criteria for SS (3 points for FS≥1 and antiSSA positivity, 1 point for UWS ≤0.1 ml/min, Schirmer ≤5 mm/5min and OSS ≥59). In some of our longstanding patients, MSG biopsies performed at disease onset scored negative and have not been repeated, either because they are judged to be unnecessary by the attending physician or due to patient refusal. While ESSDAI reflects SS biological activity, ESSPRI is claimed to be disease-specific for SS (4) and both scores have been validated (5). The aim of this study was to investigate EULAR Sjögren Syndrome Disease Activity Index (ESSDAI) and EULAR Sjögren Syndrome Patient Reported Outcome (ESSPRI) in a monocentric sicca syndrome cohort, all of whom have a working clinical diagnosis of SS.

Methods: Cross sectional study carried out at our AID Unit from January to December 2016. Inclusion criteria: 1) sicca syndrome symptoms as main complaint and clinical diagnosis of SS; 2) absence of any other known autoimmune disease (AID) at inclusion time; 3) follow up in AID Unit of at least 1 year. Demographic and clinical features, previous diagnostic investigation, ongoing therapeutics and fulfillment of sequential classification criteria were recorded. ESSDAI activity (Low <5, moderate 5 – 13, high ≥14) and ESSPRI-Patient acceptable symptom state (PASS < 5 points) (5) were performed once in each patient. Parameters were analyzed according to disease duration. Univariate statistical analysis was performed using the Wilcoxon Mann-Whitney test for non-parametric distributed data.

Results: Overall, between January and May 2017, 45 consecutive patients were included, of which 18 (40%) met 2002; 7 (16%) 2012; and 15 (33%) the 2016 SS classification criteria. Median age was 63 years (y), IQR 57-72, range 29-83; 100% female; median disease duration was 12 y, IQR 8-19; range 2-36; median ESSDAI and ESSPRI were, respectively 0, IQR 0-2; range 0-17 and 5, IQR 3,7-7, range 0 – 10. Two patients were diagnosed with lymphoma, one prior to enrolling time. Disease duration was greater than 10 y in 29 (64%) and smaller or equal to 10 y in 16 (35%) patients. Approximately half of the patients in each group were on hydroxychloroquine (200 mg/day) and ≤5 mg/day of prednisolone. Both groups presented low biological activity (only 2 patients had a score above 14, one due to renal and another due to lung involvement). There were no statistically significant demographic or clinical differences according to disease duration.

Conclusion: Regardless of disease duration, patients presented low biological activity but remained symptomatic with a PASS ≥ 5. ESSDAI and ESSPRI provide important information and therapeutic stratification, particularly as regards the need for new therapies that provide symptomatic relief.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/long-term-cohort-of-patients-with-a-clinical-diagnosis-of-sjogrens-syndrome-activity-index-and-patient-reported-outcome-performance/